Primary myeloid sarcoma of the gynecologic tract: a report of two cases progressing to acute myeloid leukemia

Primary gynecologic myeloid sarcomas are rare, and their diagnosis is often difficult. Differential diagnosis includes lymphomas and carcinomas of the gynecologic tract. We report the clinical, morphological, immunohistochemical and cytogenetic features of two cases of chloromas of the female genital tract, which progressed to acute myeloid leukemia in spite of aggressive therapy.     

Hodgkin's disease presenting as a cholestatic febrile illness: incidence and main characteristics in a series of 421 patients

 In order to determine the frequency and characteristics of patients with liver abnormalities as the presenting manifestation of Hodgkin's disease (HD), 421 consecutive HD patients were studied. Six patients in the series (1.4%) presented with liver abnormalities that led to of a liver biopsy and the subsequent diagnosis of HD. All had fever prior to HD diagnosis, four frank jaundice, and one hepatic failure. No patient had pruritus. Moderate hepatomegaly was present in four patients. Cholestasis was observed in all cases; in most patients a moderate increase in the transaminase activity was also seen. Two patients had a mild rise in the serum LDH, four had leukopenia, and one eosinophilia. At liver histologic study, Reed-Sternberg cells were demonstrated in four patients; in the remaining two, the presence of atypical histiocytes, lymphocytes, and eosinophils was highly suggestive of HD, the latter diagnosis being confirmed by subsequent study of bone marrow and/or retroperitoneal lymphadenopathies. In three of the six patients, HD was not demonstrated in sites other than the liver. Three patients older than 60 years died shortly after HD diagnosis. By contrast, three patients younger than 40 years showed a dramatic response to chemotherapy: two of them had a further relapse, and one is considered cured after 14 years of continuous remission. Liver disease constitutes an infrequent form of HD presentation which must be included in the differential diagnosis of any patient with fever of unknown origin. Received: 26 October 1995 / Accepted: 15 February 1996     

Benign liver-cell adenoma associated with long-term administration of an androgenic-anabolic steroid (methandienone).

A 19-year-old man with paroxysmal nocturnal hemoglobinuria treated for 3 years with Methandienone was admitted to the hospital with hemoperitoneum due to the rupture of an hepatic tumor. Histology revealed that it was a benign liver cell adenoma, with a pathologic appearance and mode of clinical presentation closely resembling those of cases observed to develop in association to contraceptive steroids.     

Real-time PCR genotyping of human platelet alloantigens HPA-1, HPA-2, HPA-3 and HPA-5 is superior to the standard PCR-SSP method

Genotyping of the human platelet alloantigens (HPA) is useful for the diagnosis and therapy of the patients with alloimmune thrombocytopenic syndromes, such as post-transfusion refractoriness to platelets, post-transfusion thrombocytopenic purpura and foetomaternal alloimmune thrombocytopenia. We have developed, optimized and validated a new method for simultaneous genotyping of HPAs - HPA-1, HPA-2, HPA-3 and HPA-5 - by using the real-time polymerase chain reaction (PCR) based on TaqMan technology. Its performances were compared to those of the standard PCR-sequence-specific primers (SSP) method by testing 120 DNA samples. Several discrepancies between the two methods have been observed, especially in the HPA-3 genotyping. Evidently, the PCR-SSP method produced several false positive results due to its technical drawbacks. Based on our comparison, we believe that the new real-time TaqMan PCR assay for the HPA-1, HPA-2, HPA-3 and HPA-5 genotyping is faster, more reliable and reproducible, compared to the standard PCR-SSP.     

HTLV-1 and HTLV-2 infections in HIV-infected individuals from Santos, Brazil: seroprevalence and risk factors

Because HTLV-I, HTLV-2, and HIV share identical modes of transmission, simultaneous or subsequent infections with these retroviruses are to be expected. The population of Santos, the largest port in Latin America, includes large numbers of female commercial sex workers and intravenous drug users, presumably having been exposed to retroviral infection. To evaluate the seroprevalence of HTLV infection and their associated risk factors, a cross-sectional survey was carried out in 499 HIV-infected individuals from Santos, Brazil. HTLV testing consisted of enzyme immunoassays for serologic screening and confirmatory Western blot testing. Overall HTLV-I and HTLV-2 seroprevalences were 6.0% (95% confidence interval [CI], 3.9-8.1) and 7.4% (95% CI, 5.1-9.7), respectively. Multivariate logistic regression for statistical analysis revealed HTLV-I infection to be independently associated with: intravenous drug use (IDU) (odds ratio [OR]. 2.99; 95% CI, 1.09-8.20), seropositivity to hepatitis C virus (HCV) (OR, 3.03; 95% CI, 1.02-9.01) and < 3 years of education (OR, 4.73; 95% CI, 1.56-14.41). HTLV-2 infection was associated with: IDU (OR, 3.22; 95% CI, 1.33-7.84), HCV seropositivity (OR, 5.40; 95% CI, 1.86-15.66) and nonwhite race (OR, 3.32; 95% CI, 1.58-7.00). Results indicate that HIV-infected individuals living in Santos are at similarly high risk of being exposed to HTLV-1 and HTLV-2. IDU constitutes the main risk factor for HTLV acquisition in this population, and there is no significant risk associated with sexual practice.     

Matrix-assisted laser desorption ionization

Since its invention, matrix-assisted laser desorption ionization (MALDI) has found wide application in mass spectrometry of high molecular weight compounds such as synthetic polymers and biopolymers. Despite widespread application of MALDI, the fundamental processes of ion formation and desorption are still poorly understood. The chemistry of the MALDI process, occurring both during sample preparation and during ionization is reflected in the mass spectrum. As the MALDI technique now stands a low concentration of analyte molecules, which usually exhibit only moderate absorption per molecules, is embedded in matrix crystals consisting of a small, highly absorbing species. In this manner the efficient and controllable energy transfer is retained while the analyte molecules are separated from excessive energy that would lead to their decomposition. The matrix is believed to serve two major functions: adsorption of energy from the laser light and the isolation of analyte molecules from each other. There are 3 major methods for the preparation of samples for analysis which are quite quick and simple: dried droplet, surface and sandwich preparation. Experiments with pH indicator dyes serve as proof that analyte's charge state in the matrix crystals is the same as in solution. Upon laser desorption a sudden and explosive phase transition occurs and a dense plume of desorbed material is formed. The initial velocity of analyte ions in the plume depends only on the matrix used. Initial species formed as a result of laser desorption are tiny clusters. They consist of a matrix, analyte and other ionic species embedded in the matrix crystals all held together by hydrogen bonds and coulombic interactions. The first essential charging and thus ionization process is the statistical occurrence of clusters with a deficit/excess of anions of cations. Very small initial clusters are likely to be highly charged. Highly charged initial clusters cannot survive in the matrix plume and their charge drops. Clusters shrink by evaporation of neutral molecules. This paper presents only those cases leading to analyte ions and compares MALDI and the electrospray ionization technique.